Arteriovenous malformations (AVMs) within the spinal canal and in the paraspinal region are unusual. Spinal cord and dural AVMs or arteriovenous fistulas have been the subject of numerous reports, but paraspinal malformations causing venous congestion or hemorrhage in the spinal canal are rare and present special diagnosis and management challenges. The authors review previously published reports on 16 children with paraspinal AVMs. They also describe the 17th case of a child with a paraspinal AVM who presented with a spontaneous spinal epidural hematoma. To the best of the authors' knowledge, there has been no other case of a spinal epidural hematoma associated with a paraspinal AVM. In each of the 17 cases, the vascular lesion was successfully obliterated using endovascular therapy. Embolization with permanent occlusive agents is an effective treatment for these rare but potentially debilitating lesions.